We report a case of renal cell carcinoma (RCC) with extensive oncocytoid features developing in a 12-year-old African-American boy after chemotherapy for cardiac leiomyosarcoma at 3 months of age. The tumor showed histopathologic features similar to those previously reported in RCC after chemotherapy for neuroblastoma and now considered a specific entity in the World Health Organization classification of renal tumors as "RCC associated with neuroblastoma." Our report expands the spectrum of tumors (beyond neuroblastoma) in which RCCs with such an appearance can arise in the pediatric age group after chemotherapy.